Ear and Hearing Considerations For Children with Down Syndrome

Douglas K. Holmes, MD, FACS

Randi Leonard Holmes, MS, CCC-A, Audiologist

ENT & Audiology Associates, Raleigh, NC

Introduction

As an ear, nose, and throat surgeon and an audiologist, caring for the ears and hearing of children with Down syndrome is among the most challenging but yet rewarding aspects of our practice. Special considerations of anatomical differences (such as small ear canals, alterations in the skull base causing Eustachian tube dysfunction) and unique physiology (such as Atlantoaxial Instability, middle ear and inner ear hearing loss) need to be taken to insure successful outcomes. A normally straightforward procedure such as placing tubes and removing adenoids requires special equipment, skill, and resolve to deal with the small ear canals and special X-ray studies and positioning on the operating room table to avoid problems of cervical spine instability. But the effort to improve hearing and reduce the number of ear infections is more than made up as we watch the children grow and develop successfully to their maximum potential, unimpeded by hearing loss and repeated ear infections.

External ear canal

The ear canals of children with Down syndrome are almost universally much smaller and more narrow. This allows cerumen (wax) to block the canals and make examinations and procedures more difficult. At times general anesthesia in the hospital operating room is required to clean and inspect the ears.

Tympanic membrane (TM, eardrum), middle ear, Eustachian tube

Children with Down syndrome have poor Eustachian tube function leading to chronic middle ear fluid and hearing loss. The Eustachian tube is designed to ventilate the middle ear;  in the majority of children with Down syndrome, it must be bypassed via the use of small tympanostomy tubes (pressure equalizing or PE tubes) which straddle the eardrum and allow air to pass into the middle ear. Unfortunately the tubes stay in place only for only six to nine months and may need replacement.

Hearing evaluation

Taken verbatim from an article in Down Syndrome Quarterly, volume 2, number 4, December 1997, pages 1-4 by Nancy Roizen, MD, University of Chicago:

The majority of children with Down syndrome have some type of hearing loss. Therefore, all children with Down syndrome must have the status of their hearing determined within the first few weeks of life. Children without a hearing loss on the first evaluation still need continued monitoring in light of the high incidence of conductive hearing loss in relation to middle ear effusions. Children with hearing losses need a clarification of the type of hearing loss (conductive, sensorineural, or mixed) and treatment of the hearing loss medically with antibiotics and surgically with tympanostomy tubes. Because of the persistence of middle ear problems, the frequency of extremely small external ear canals, and the need for open external canals free from cerumen, many children with Down syndrome need to be monitored every three months by an otolaryngologist. Many cases of hearing loss including both sensorineural and conductive types are responsive to the therapy. Hearing aids need to be considered. Educational intervention and speech therapy are extremely important. Nothing less than aggressive management of hearing problems in children with Down syndrome is acceptable.

We agree most heartily with Dr. Roizen, especially in the need for aggressive management, i.e. doing all that is possible to correct any hearing loss, not to pass off any hearing loss as inconsequential, and not to assume that the hearing loss will correct itself over time.

Our current recommendations are as follows:

· Meticulous otologic examinations, with cleaning of cerumen under general anesthesia in the O.R. as necessary

· In the first month of life, referral to a pediatric otolaryngologist and pediatric audiologist for complete and comprehensive medical and audiometric evaluation including tympanometry, acoustic reflex testing, and formal ABR testing. Continued follow-up every 2 to three months is a must.

· Routine monitoring of middle ear effusions and prompt appropriate antibiotic treatment is recommended. If the effusions persist, then tubes should be placed.

· Hearing aid amplification should be provided if there is an uncorrectable conductive hearing loss or if a sensorineural loss is detected.

Upper airway obstruction

The craniofacial characteristics of children with Down syndrome (flat skull base, mid-face narrowing) are responsible for the increased incidence of nasal blockage and discharge, obstructive sleep apnea, and mouth breathing. Enlargement of tonsils and adenoids may also block the upper airway and lead to the need for tonsillectomy and adenoidectomy (T&A). Unfortunately T&A does not lead to as dramatic relief as in children without Down syndrome. If T&A is to be performed, then x-rays of the neck to rule out instability of the cervical spine (Atlantoaxial Instability) must be performed.